Abnormal fatty acid metabolism in spinal muscular atrophy may predispose to perioperative risks.

Journal Article (Journal Article)

A 15 year old boy with SMA type II underwent spinal fusion and suffered a mitochondrial Reye-like catabolic crisis 4 days postop with hypoketotic hypoglycemia, lactic acidaemia, hyperammonemia and liver failure, with 90% coagulative necrosis and diffuse macro- and microvesicular steatosis, requiring orthotopic liver transplantation. This crisis responded in part to mitochondrial therapy and anabolic rescue. He made a dramatic sustained neurological recovery, though his post-transplant liver biopsies revealed micro- and macrosteatosis. We hypothesize that a combination of surgical stress-catecholamine induced lipolysis, prolonged general anaesthesia with propofol and sevoflurane, and perioperative fasting on a background of decreased β-oxidation were potential risk factors for the mitochondrial decompensation.

Full Text

Duke Authors

Cited Authors

  • Zolkipli, Z; Sherlock, M; Biggar, WD; Taylor, G; Hutchison, JS; Peliowski, A; Alman, BA; Ling, SC; Tein, I

Published Date

  • September 2012

Published In

Volume / Issue

  • 16 / 5

Start / End Page

  • 549 - 553

PubMed ID

  • 22264649

Electronic International Standard Serial Number (EISSN)

  • 1532-2130

Digital Object Identifier (DOI)

  • 10.1016/j.ejpn.2012.01.004


  • eng

Conference Location

  • England